Pathogenic FBN1 variants in familial thoracic aortic aneurysms and dissections.

Marfan syndrome (MFS) due to mutations in FBN1 is a known cause of thoracic aortic aneurysms and acute aortic dissections (TAAD) associated with pleiotropic manifestations. Genetic predisposition to TAAD can also be inherited in families in the absence of syndromic features, termed familial TAAD (FTAAD), and several causative genes have been identified to date. FBN1 mutations can also be identified in FTAAD families, but the frequency of these mutations has not been established. We performed exome sequencing of 183 FTAAD families and identified pathogenic FBN1 variants in five (2.7%) of these families. We also identified eight additional FBN1 rare variants that could not be unequivocally classified as disease-causing in six families. FBN1 sequencing should be considered in individuals with FTAAD even without significant systemic features of MFS.

Contemporary management of blunt aortic trauma.

Blunt thoracic aortic injury (BTAI) remains a common cause of death following blunt mechanisms of trauma. Among patients who survive to reach hospital care, significant advances in diagnosis and treatment afford previously unattainable survival. The Society for Vascular Surgery (SVS) guidelines provide current best-evidence suggestions for treatment of BTAI. However, several key areas of controversy regarding optimal BTAI care remain. These include the refinement of selection criteria, timing for treatment and the need for long-term follow-up data. In addition, the advent of the Aortic Trauma Foundation (ATF) represents an important development in collaborative research in this field.

The reduction of dose in paediatric panoramic radiography: the impact of collimator height and programme selection.

OBJECTIVES: The aim of this work was to estimate the doses to radiosensitive organs in the head of a young child undergoing panoramic radiography and to establish the effectiveness of a short collimator in reducing dose. METHODS: Thermoluminescent dosemeters were used in a paediatric head phantom to simulate an examination on a 5-year-old child. The panoramic system used was an Instrumentarium OP200 D (Instrumentarium Dental, Tuusula, Finland). The collimator height options were 110 and 140 mm. Organ doses were measured using exposure programmes intended for use with adult and child size heads. The performance of the automatic exposure control (AEC) system was also assessed. RESULTS: The short collimator reduced the dose to the brain and the eyes by 57% and 41%, respectively. The dose to the submandibular and sublingual glands increased by 32% and 20%, respectively, when using a programme with a narrower focal trough intended for a small jaw. The effective dose measured with the short collimator and paediatric programme was 7.7 µSv. The dose to the lens of the eye was 17 µGy. When used, the AEC system produced some asymmetry in the dose distribution across the head. CONCLUSIONS: Panoramic systems when used to frequently image children should have programmes specifically designed for imaging small heads. There should be a shorter collimator available and programmes that deliver a reduced exposure time and allow reduction of tube current. Programme selection should also provide flexibility for focal trough size, shape and position to match the smaller head size.

Effects of intraoperative steroid injection on the outcome of pterygium surgery.

PURPOSE: To evaluate the effects of intraoperative triamcinolone injection on the outcome of pterygium surgery. METHODS: This prospective study included 54 eyes with primary nasal pterygia that underwent pterygium surgery with a bare-sclera technique and intraoperative mitomycin C application. Patients were randomized into two groups; the steroid group that received subconjunctival injection of 12 mg triamcinolone acetonide at the end of surgery, and the control group that did not receive such steroid injection. Main outcome measures included presence of conjunctival inflammation at 1 month postoperatively as well as recurrence of pterygium. RESULTS: Twelve-month follow-up was completed in 48 eyes (23 in the steroid group and 25 in the control group). At 1 month postoperatively, different grades of conjunctival inflammation were present in 11 (47.8%) of the steroid group and in 14 (56%) of the control group (P=0.39). For eyes with moderate or severe postoperative inflammation, subconjunctival triamcinolone was injected; these included 6 (26.1%) and 9 (36%) in the steroid and control groups, respectively (P=0.54). During follow-up, surgical area showed fine episcleral vessels without fibrous tissue in 1 (4.3%) of the steroid group and 3 (12.0%) of the control group (P=0.33), which all regressed after triamcinolone injection. Conjunctival recurrence of pterygium was seen in 2 (8.7%) of the steroid group and in 1 (4.0%) of the control group (P=0.47). No eye developed corneal recurrence in either group. CONCLUSIONS: In pterygium surgery with a bare-sclera technique and mitomycin C application, intraoperative triamcinolone injection did not significantly reduce postoperative conjunctival inflammation or pterygium recurrence.

Operative outcomes using a side-branched thoracoabdominal aortic graft (STAG) for thoraco-abdominal aortic repair.

BACKGROUND: Pre-manufactured branched grafts now allow an endovascular approach to the repair of thoraco-abdominal aortic aneurysm (TAAA) with visceral vessels' involvement. Similar grafts have been employed in open surgery, generally as a second choice for TAAAs, which are not amenable to patch/inclusion technique for visceral vessel attachment. Results with branched grafts have not been reported in series of open TAAA repairs. The purpose of this report is to describe perioperative risks and outcomes in a consecutive series of patients with pre-manufactured side-branched thoracoabdominal aortic grafts (STAGs) for surgical TAAA repair. METHODS: Between 1996 and 2009, pre-manufactured STAGs were used in 50 patients with TAAA that required reattachment of the visceral and renal arteries. Operative details, perioperative mortality and ischaemic complications were examined. RESULTS: Mean age was 53 years; 18 patients were females. The cases included redo (n = 24), patients affected by genetic disorder (Marfan) (n = 20) and patients with aortic dissection (n = 27). The mean clamp time was 84.1 min. Perioperative mortality was 12.0% (6/50). Neurologic deficits occurred in 2% (1/50). Postoperative renal dysfunction was detected in 19 patients (38%). CONCLUSION: The use of a STAG produced acceptable mortality, bowel and neurological ischaemic risks. Improved strategies to prevent renal ischaemia before and during repair of TAAA with visceral involvement are needed.

Serum myoglobin and renal morbidity and mortality following thoracic and thoraco-abdominal aortic repair: does rhabdomyolysis play a role?

OBJECTIVES: The intractability of renal dysfunction following thoracic and thoraco-abdominal aortic repair leads us to believe that the accepted mechanisms of renal injury - ischaemia and embolism - are incompletely explanatory. We studied postoperative myoglobinaemia and renal dysfunction following aortic surgery. METHODS: Between September 2006 and February 2008, we studied serum myoglobin in 109 patients requiring thoracic/thoraco-abdominal repair for three postoperative days. Forty-two of the 109 (38%) patients were female. The median age was 67 years (range 23-84 years). As we have focussed more attention on renal function, our independent renal consultants have dialysed more aggressively. We divided dialysis into: (1) creatinine indication, (2) non-creatinine indication and (3) no dialysis. RESULTS: Thirteen of the 109 (12%) patients met creatinine indication for dialysis (>4 mg dl(-1)) and an additional 28 (26%) were dialysed for other reasons. Overall mortality was 12 out of 109 (11%) cases: 11 out of 41 (27%) in dialysed patients and one out of 68 (1.5%) in non-dialysed patients. Mortality did not differ between the indications for dialysis. Predictors of mortality were baseline glomerular filtration rate (GFR), postoperative myoglobin and dialysis. The only predictor of dialysis was postoperative myoglobin. CONCLUSION: A strong relationship between postoperative serum myoglobin and renal failure suggests a rhabdomyolysis-like contributing aetiology following thoraco-abdominal aortic repair. We postulate a novel mechanism of renal injury for which mitigation strategies should be developed.

[Hematogenous osteomyelitis of the calcaneus in children: 26 cases]. / L'ostéomyélite hématogène du calcanéum chez l'enfant: à propos de 26 cas.

PURPOSE OF THE STUDY: Osteomyelitis is rarely observed in the calcaneus; about 3 to 10% of the bone infections in children. The diagnosis is often established late because of the less pronounced symptoms in long-bone localizations. We report a series of 26 cases of osteomyelitis of the calcaneus observed in children. MATERIAL AND METHODS: We studied the clinical history, the diagnostic process and the treatments delivered. Outcomes were assessed in terms of complications, anatomy and function at mean two years follow-up (range one to seven years). RESULTS: There were 15 boys and 11 girls, mean age was seven years (range one month to 13 years). Mean time from symptom onset to consultation was 13 days and mean time from consultation to hospital admission was four days, range one to 29 days. The clinical presentation was not specific. Body temperature was not above 38.5 degrees C in 45% of patients. Symptoms were fever, pain in the rear foot and functional impotency of the lower limb. Eight patients (30%) complained of moderate pain, 18 (70%) of intense pain. The pain was focused far from the calcaneus in six patients, retarding the diagnosis. Laboratory tests did not always reveal signs of inflammation. White cell counts above 10,000 were noted in only 61% of patients. The diagnosis of osteomyelitis of the calcaneus was based on: the plain X-ray, which revealed a defect in the calcaneus (n=12), ultrasound (performed in 19 patients) which revealed calcaneal subperiosteal detachment (n=6), collections in the rear foot (n=3) and soft-tissue thickening (n=4). Bone scintigraphy was performed in one child and showed intense uptake in the calcaneus. Magnetic resonance imaging, performed in one patient, demonstrated an anomalous signal in the calcaneus (high-intensity T(2) and low-intensity T(1) with presence of a subperiosteal abscess). Bacteriology was positive in 53% of the children. Medical treatment was delivered for all patients and 23 underwent a surgical procedure. For one of the three patients treated medically, the diagnosis of osteomyelitis of the calcaneus was clinical, since the plain X-ray was normal, the ultrasound yielded no evidence of abscess formation and the bacteriology was negative; but after two months of antibiotic treatment, bone remodelling was in favour of osteomyelitis of the calcaneus. For the two other patients treated medically, the plain X-ray showed a defect in the calcaneus, which had filled after two months of antibiotics. For the 23 patients treated surgically, the procedure was an evacuation of a subperiosteal abscess for 13 (n=6 nonruptured and 7 ruptured). Surgery revealed a bone lesion in nine children allowing curettage of the defect. Articular involvement was noted in eight cases: subtalar osteoarthritis (n=6) and tibiotarsal arthritis (n=2). Two surgical explorations failed to find any abscess formation; blood cultures confirmed the diagnosis and enabled isolation of the causal germ. Outcome was assessed with a mean follow-up of two years, range one to seven years. Nineteen patients (73%) were free of sequelae. Seven patients (27%) presented poor outcome with significant limitation of motion in the rear foot and ankle ankylosis. Seven patients developed chronic fistules, with persistent discharge at last follow-up. The poor results were observed in patients treated late with mean 17 days before consultation. Six of the seven cases of poor outcome were associated with arthritis involving a calcaneal joint (subtalar and tibiotarsal in two patients and subtalar in four). DISCUSSION: The same pathophysiological phenomenon as observed in long-bone localizations is noted for osteomyelitis of the calcaneus; the calcaneus has an apophysis, which is equivalent to the metaphyseal region of long bones, leading to the bone's vulnerability to hematogenous infection. Late diagnosis can be related to the notion of trauma, the manifestations of osteomyelitis being attributed to ligament injury. The positive diagnosis of osteomyelitis of the calcaneus is often established late because of late consultation (13 days in our series) or the minimal expression of general signs. Magnetic resonance imaging contributes significantly to diagnosis by showing an abnormal bone signal; it can also disclose associated abscess formation. Authors differ in their descriptions of the complications. The analysis of our results shows that the prognosis of osteomyelitis of the calcaneus is related to early diagnosis and management. Associated septic arthritis is an element of poor prognosis.

[Acute hematogenous osteomyelitis of the obturator rim in seven children]. / L'ostéomyélite aiguë hématogène du pourtour du foramen obturé chez l'enfant : à propos de sept cas.

PURPOSE OF THE STUDY: Acute osteomyelitis of the rim of the obturator foramen is rarely reported. The clinical presentation is atypical, making diagnosis a difficult task. We report a series of seven cases of osteomyelitis of the obturator rim which illustrate the different features of this localization. MATERIAL AND METHODS: The series included seven boys with osteomyelitis involving the obturator foramen. We reviewed the clinical history, the diagnostic approach and management. Complications and anatomic outcome were noted at mean three years follow-up (range one to 10 years). RESULTS: Mean age was 9.5 years (five to 12 years). Bilateral involvement was noted in one boy. Mean time from symptom onset to consultation was five days and mean time from consultation to hospitalization was eight days. No specific clinical presentation could be identified. Fever was not a constant feature. Mean body temperature was 38.6 degrees C and was not greater than 38.5 degrees C in four children. Symptoms were limited to hip pain in five cases and abdominopelvic pain was noted in two. Physical examination failed to trigger exquisite ischial or pubic pain in two patients. The osteomyelitis involved the ischiopubic ramus in four cases, the ischium in two with one bilateral case, and the pubis in one. Certain diagnosis was established as follows: MRI findings plus isolation of the pathogenic agent (n=4) ; the plain x-ray showed a defect in the ischiopubic ramus, MRI showed signs favoring osteomyelitis of the ischiopubic ramus and surgery evacuated a purulent collection (n=1) ; strong uptake of the obturator rim on scintigraphy (n=2). Medical treatment was given in all cases and surgery was performed in six patients. Outcome was noted at three years follow-up, range one to 10 years. All patients recovered normal function. There were no complications. DISCUSSION: Hematogenous osteomyelitis of the pelvis is exceptional (2-11 % of cases of osteomyelitis). Localization in the obturator rim is rare. We discuss the specific diagnostic and therapeutic features of this localization. Despite the controversy concerning the treatment of pelvic osteomyelitis and in particular cases involving the obturator rim, prognosis is generally good. No complications have been described in the literature specifically associated with osteomyelitis of the obturator rim.

[Acute hematogenous osteomyelitis of the neck of the femur in children: 28 cases]. / L'ostéomyélite aiguë hématogène du col du fémur chez l'enfant : à propos de 28 cas.

PURPOSE OF THE STUDY: Osteomyelitis of the neck of the femur is uncommon, often with a misleading clinical presentation. We report a series of 28 cases of osteomyelitis of the femoral neck to illustrate the particular clinical and imaging findings related to this localization. MATERIAL AND METHODS: This was a series of 28 children treated in our unit from 1990 through 2004: 17 boys and 11 girls, mean age eight years (range one month to 14 years). We analyzed the data in this series using a standard checklist, which noted the diagnostic and therapeutic measures. Results were analyzed by studying the complications, anatomic and functional outcome at mean follow-up of 3.5 years. RESULTS: Time from symptom onset to consultation was five days on average with a delay of 4.5 days from consultation to hospitalization. Intense pain was noted for eight patients (28%) and total functional incapacity of the limb was noted for 15 (53%). Hip stiffness was observed in 11 patients (39%). The diagnosis of osteomyelitis of the femoral neck was established on the basis of imaging (MRI or scintigraphy) in three patients with bacteriological proof in two, of operative findings which confirmed the neck localization in 19, and on changes in the X-ray image of the neck in six. A positive bacteriology was noted in 71%. The germ was isolated from blood cultures and local samples obtained by arthrotomy in five patients (same germ), only in blood cultures for four, and only in local samples in 11. Met-S Staphylococcus aureus was isolated in 18 patients, Met-R S. aureus in one and a Streptococcus in one. All patients were given medical treatment and 25 underwent surgery. There were five thromboembolic complications and five patients who developed femoral pandiaphysitis. Results were analyzed at mean 3.5 years (range four months to 14 years). Complete hip motion was recovered in 78%. There were four cases of hip stiffness and two cases of ankylosis. Partial cephalic necrosis was noted in two hips and total necrosis of the head and neck in two. DISCUSSION: Little data is available in the literature on isolated osteomyelitis of the femoral neck. Based on the pathogenic mechanisms known for osteomyelitis, an isolated localization in the neck of the femur, with no other site in the hip joint, is quite possible in an early stage of infection. We discuss the specific clinical and imaging features of this localization. Analysis of our findings show that the prognosis of femoral neck osteomyelitis is directly related to time to management. Outcome is poorer when treatment is started late. Prognosis is poor if pandiaphysitis develops.

Robinow syndrome: report of two cases and review of the literature.

We report two patients with Robinow syndrome, review the published literature and stress the importance and limitations of radiographic examination in the diagnosis of this disorder, which shows extreme clinical and radiographic variability. The radiographic differential diagnosis of Robinow syndrome is discussed.

[Fracture of the ulna and bilateral congenital dislocation of the head of the radius: A case report]. / Fracture de l'ulna et luxation congénitale bilatérale de la tête radiale: à propos d'un cas.

We report a case of bilateral congenital dislocation of the radial head discovered at the time of a fracture of the ulna. The dislocation was missed initially and when considered as part of the Monteggia fracture, raised a problem concerning the therapeutic approach. The purpose of this report is the emphasize the importance of careful physical examination before undertaking treatment of any lesion.

Thoracic aortic aneurysms.

Remarkable progress has been made in the surgical treatment of thoracoabdominal aortic aneurysms. The decline in mortality and complication rates can be attributed to improvements in perioperative care and in surgical technique, particularly the adoption of adjunct distal aortic perfusion and cerebrospinal fluid drainage. Neurologic deficit is no longer a major threat to patients, as the use of adjuncts has brought the incidence down to 2.4% for all thoracoabdominal aortic aneurysms. However, we continue to pursue research to improve organ preservation, particularly for the most troublesome extent II thoracoabdominal aortic aneurysm.

Number needed to treat: analyzing of the effectiveness of thoracoabdominal aortic repair.

BACKGROUND: Number needed to treat (NNT) is a method used to calculate the number of patients who need to be treated to prevent one adverse outcome. To analyze the effectiveness of thoracoabdominal and descending thoracic aortic aneurysm repair, we computed the NNT required to prevent one death. METHODS: Between Jan 1991 and Feb 2003, we repaired 1004 aneurysms of the descending thoracic and thoracoabdominal aorta. We followed the patients from surgery until death. Five-year actuarial survival in our population was computed by the Kaplan-Meier method. Natural history data for comparison were taken from the population-based work of Bickerstaff et al., 1982. NNT was calculated as the reciprocal of the risk difference at 5 years. 95% confidence intervals were computed by the method of Daly. RESULTS: Five-year mortality in the population-based cohort was 87 vs. 39% in our treated population, for a risk difference of 48%. 1/0.48=2, indicating that two patients need to be treated to prevent one death at 5 years (95% CI 1.8-2.5, p<0.0001). CONCLUSION: An NNT of two demonstrates the effectiveness of surgical repair of descending thoracic and thoracoabdominal aortic aneurysms when compared to the natural history. By comparison, carotid endarterectomy for symptomatic lesions >70% has an NNT of 15 to prevent a single stroke or death. NNT can also be applied to aneurysm size criteria to estimate the effort required to prevent death or rupture for a given aneurysm size.

[Spinal anesthesia in children: comparative study of hyperbaric bupivacaine with or without clonidine]. / La rachianesthésie chez l'enfant: étude comparative de la bupivacaïne hyperbare avec et sans clonidine.

OBJECTIVE: To evaluate the effect of intrathecal clonidine in children. STUDY DESIGN: A prospective randomised study. PATIENTS AND METHODS: 45 children, 6 to 15 years old, were randomised in two groups; receiving either 0.5% hyperbaric bupivacaine or 0.5% hyperbaric bupivacaine added to clonidine 2 micrograms.kg-1. We assessed quality and length of motor and sensory blocks and side effects of clonidine: hypotension, bradycardia and sedation. RESULTS: Clonidine was associated with prolongation of motor block. 190 +/- 42 min vs 150 +/- 35 min (p < 0.01), but the difference was not significant. Postoperative analgesia was longer in clonidine group, 490 +/- 35 min vs 200 +/- 50 min (mean +/- SD), p < 0.001. Clonidine was associated with higher incidence of hypotension 54 vs 36% and bradycardia 30 vs 0%. CONCLUSION: These data suggest that intrathecal clonidine 2 micrograms.kg-1 is associated with extending duration of postoperative analgesia but with moderate side effects.

Determinants of hospital length of stay after thoracoabdominal aortic aneurysm repair.

PURPOSE: Extended hospital length of stay (LOS) and consequent high costs are associated with thoracic and thoracoabdominal aortic aneurysm (TAAA) surgery. In this study, we examined factors that may influence LOS after TAAA repair. METHODS: Five hundred forty thoracic and TAAA repairs were performed by one surgeon between 1990 and 1999. The data were analyzed with multiple linear regression with appropriate logarithmic transformation. The predictor variables included patient demographics, disease extent, severity indicators, intraoperative factors, and postoperative complications. RESULTS: The median LOS was 15 days. Postoperative creatinine level of greater than 2.9 was the most important predictor of LOS, followed by spinal cord deficit, age, and pulmonary complication (all statistically significant with P <.05). A second model constrained to preoperative risk factors showed both age and complete diaphragmatic division to be associated with increased LOS. Preservation of the diaphragm led to reduced LOS by an average of 4 days. The adjunct cerebrospinal fluid drainage and distal aortic perfusion was associated with a decrease in LOS, although it did not reach statistical significance. CONCLUSION: Renal failure, spinal cord deficit, and pulmonary complication were the major determinants of LOS in patients for TAAA repair. This study shows that the preservation of diaphragmatic function and the use of the adjunct distal aortic perfusion and cerebrospinal fluid drainage may reduce hospital LOS.

Chronic aortic dissection not a risk factor for neurologic deficit in thoracoabdominal aortic aneurysm repair.

OBJECTIVE: chronic aortic dissection has long been considered a risk factor for neurologic deficit following thoracoabdominal aortic aneurysm (TAA) surgery. We reviewed our experience with regard to aneurysm extent and the use of adjunct, (distal aortic perfusion/cerebrospinal fluid drainage), and examined the impact of these factors on neurologic deficit among chronic dissection and non-dissection cases. METHODS: between February 1991 and March 2001, we repaired 800 aneurysms of the descending thoracic and thoracoabdominal aorta. Seven hundred and twenty-nine cases were elective; 196 chronic dissection, 533 non-dissection. 182/729 (24.9%) were TAA extent II. Among these, 61/182 (33%) involved chronic dissection. Adjunct was used in 507/729 (69.6%). We conducted detailed multivariate analyses to isolate the impact of chronic aortic dissection on neurologic morbidity, with other important risk factors taken into account. RESULTS: overall, 32/729 (4.4%) patients had neurologic deficit upon awakening; 7/196 (3.6%) in chronic dissections, and 25/533 (4.7%) in non-dissections. Adjunct had a major effect, reducing neurologic deficit in TAA extent II from 10/36 (27.8%) to 10/146 (6.9%) (p=0.001). However, in univariate and multivariate analysis, chronic dissection did not increase the risk of neurologic deficit, regardless of extent or mode of treatment. CONCLUSION: in contrast to previous reports, we determined that chronic aortic dissection is not a risk factor in TAA patients.

[Nocardia farcinica brain abscess: clinical and specific radiological findings and management. Report of two cases in immunononcompromised patients]. / Abcès cérébraux à nocardia farcinica: spécificités cliniques, radiologiques et thérapeutiques. A propos de deux cas chez des patients immunocompétents.

Nocardia spp. cerebral abscesses are rare and usually occur in immunocompromised patients. We report two recent cases of cerebral abscesses due to Nocardia farcinica in immunocompetent patients and review the literature about diagnosis and therapeutic issues. Outcome was good for the two patients following an early identification of the bacteria. Stereotactic biopsy was performed in one case and craniotomy with excision of the abscess in the other case. In both cases, complete identification of the bacteria could be achieved, followed by prolonged antibiotic therapy. Exposure to the germ (mainly telluric) is often difficult to suspect from the past medical history of the patient. Diagnosis is also unexpected. Despite the typically and characteristic aspect on CT and MRI, specific identification and anti-microbial sensitivity profiles are necessary to optimize treatment. In some rare cases, unusual species like Nocardia farcinica, can be resistant to numerous antibiotics requiring adjustments of medical management. Early identification of the bacteria is necessary to achieve good outcome in immunocompetents patients.

Staged repair of extensive aortic aneurysms: morbidity and mortality in the elephant trunk technique.

BACKGROUND: Extensive aortic aneurysms (ascending aorta, aortic arch, and descending or thoracoabdominal aorta) require innovative surgical techniques. Some surgeons advocate a single procedure with long periods of profound hypothermia, whereas others use a staged approach. We adopted a two-staged procedure (elephant trunk technique) in 1991 for elective repair of extensive aortic aneurysms. METHODS AND RESULTS: Between February 1991 and May 2000, we performed a total of 1146 aortic aneurysm operations. Of these, 182 (15.9%) operations were first- or second-stage elephant trunk procedures, performed in a total of 117 patients. Stage 1 was completed in all 117 patients. Stage 2 was completed in 65 (55.6%) of 117 patients. Thirty-day mortality rate for the first stage was 5.1% (6 of 117). Mortality rate during the interval between operations was 3.6% (4 of 111), of which 75% (3 of 4) were the result of aneurysm rupture. Thirty-day mortality rate for the second stage was 6.2% (4 of 65). A total of 43 patients did not return for second-stage repair. Among these patients, within an average period of 3.4 years (range, 1.5 months to 4.9 years), 13 of 43 (30.2%) died, 4 of 13 (30.8%) as the result of rupture. Two of 117 (1.7%) first-stage patients had postoperative stroke. No spinal cord dysfunction occurred in second-stage patients. CONCLUSIONS: Extensive aortic aneurysms can be repaired with acceptable morbidity and mortality rates through the use of the elephant trunk technique. Death was most commonly the result of rupture, both in interval patients awaiting scheduled second-stage repair and in patients who did not return. After the first stage, prompt treatment of the remaining segment is crucial to the success of staged repair.